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Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis (GPA) is a rare form of vasculitis that restricts blood flow.

Also called Wegener’s granulomatosis, GPA produces nodules of inflamed tissue around blood vessels. Sometimes sores develop in the respiratory tract or the kidneys, which can have serious consequences.

There are many early symptoms of GPA, including the following:

  • Frequent sinusitis
  • A lingering fever
  • Night sweats
  • Fatigue
  • Nose bleeds
  • Chronic ear infections

Granulomatosis with polyangiitis (GPA): what you should know

  • Other symptoms may include the following:
    • Joint pain
    • Loss of appetite, weight loss
    • Blood in the urine
    • Eye problems, such as conjunctivitis
    • Chest pain
    • Coughing
    • Shortness of breath and wheezing
    • Weakness
  • It is not known what triggers the immune system to overproduce the antibodies causing GPA; however, it may be a combination of an infection and genetic predisposition. 
  • With treatment, most patients recover; however, the disease returns in about half of all patients. In these cases, the disease usually comes back within two years of stopping treatment.
  • Complications, including kidney failure, usually result from lack of treatment. If untreated, kidney failure and death occur in more than 90 percent of patients.

How can I get help for granulomatosis with polyangiitis (GPA)?

Diagnosis. The sooner the diagnosis is made, the better your outcome will be. If you have any of the symptoms above, see your primary care doctor.

Your doctor will order a set of tests, including one or more of the following:

  • Blood tests to look for specific antibodies
  • Urine tests to look for signs of kidney disease
  • Chest X-rays to examine the lungs
  • Biopsy to get a sample of the lungs, skin, kidneys or nerves for analysis

Care for granulomatosis with polyangiitis at Rush

If you are diagnosed with GPA or your doctor suspects you have it, you will be referred to the Division of Rheumatology.

Medication. Treatment focuses on controlling your immune system with medications. Immunosuppressive medications can cause serious side effects and require careful monitoring.

There are two stages of treatment. The induction phase lasts three to six months, using some the following strong medicines:

  • Corticosteroids to control inflammation and the immune system
  • Cytoxan to suppress the immune system
  • Rituxan to control your immune response

The maintenance phase lasts two years or more. You will taper off of the above medications and/or add other drugs to help to control the disease, such as the following:

  • Methotrexate to decrease the activity of the immune system
  • Azathioprine to suppress the immune system

Your care team may also give you other medicines:

  • Bisphosphonate to prevent bone loss caused by some steroids
  • Trimethoprim or sulfamethoxazole to prevent lung infection

Why choose Rush for granulomatosis with polyangiitis care

  • Rush offers an onsite laboratory, infusion therapies and vaccinations. This makes your care easily coordinated and more convenient for you.
  • Experts at the Rush Vasculitis Clinic specialize in identifying, diagnosing and treating all forms vasculitis, including granulomatosis with polyangiitis (GPA).

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